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肢体-体壁复合体——该实体的历史及我们系列病例的呈现

Limb body wall complex - the history of the entity and presentation of our series of cases.

作者信息

Gulczyński Jacek, Świątkowska-Freund Małgorzata, Paluchowski Piotr, Hermann-Okoniewska Blanka, Iżycka-Świeszewska Ewa

机构信息

Department of Pathology and Neuropathology, Medical University of Gdansk, Poland.

Department of Obstetrics, Medical University of Gdansk, Poland.

出版信息

Pol J Pathol. 2019;70(1):33-41. doi: 10.5114/pjp.2019.84460.

Abstract

We present an analysis of two first historically documented limb body wall complex (LBWC) cases and our own contemporary perinatal autopsy series of this rare complex. So far it was supposed that the first case of this complex was reported in 1685 by Paul Portal. Studying the Joachim Oelhaf's autopsy report from 1613 with attached engraving showing the neonate with multiple birth defects led our research team to a conclusion that it was genuinely the first description of LBWC in the medical literature so far. We compared the Oelhaf's case from 1613 and the Portal's autopsy report from 1685 with our series of LBWC cases dissected in the Medical University of Gdansk between 1999 and 2011. Reviewing 1100 autopsy reports performed we encountered 9 cases of this unique complex. The analysis was supported by the literature review.

摘要

我们对两例历史上首次有文献记载的肢体-体壁复合体(LBWC)病例以及我们自己当代对此罕见复合体进行的围产期尸检系列进行了分析。到目前为止,人们认为该复合体的首例病例是由保罗·波塔尔于1685年报告的。研究约阿希姆·奥尔哈夫1613年的尸检报告及所附的显示患有多种出生缺陷的新生儿的雕刻图,使我们的研究团队得出结论,这是迄今为止医学文献中对LBWC的首次真实描述。我们将1613年的奥尔哈夫病例和1685年的波塔尔尸检报告与我们在1999年至2011年期间于格但斯克医科大学解剖的LBWC病例系列进行了比较。在查阅的1100份尸检报告中,我们遇到了9例这种独特的复合体病例。该分析得到了文献综述的支持。

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